Transient Pseudohypoaldosteronism Secondary to Posterior Urethral Valves - A Case Report and Review of the Literature

Abstract

In transient pseudohypoaldosteronism (TPHA), renal tubular resistance to aldosterone is thought to be secondary to renal disease. We report a case of TPHA caused by posterior urethral valves associated with urinary tract infection and review 62 cases previously reported. The infant presented with unspecific signs of vomiting and dehydration, so that pyloric stenosis was first suspected. Laboratory data and retroperitoneal sonography led to the diagnosis TPHA. This case illustrates that urine culture and renal ultrasonography should be performed in any infant with electrolyte disturbances to exclude infection or obstructive uropathy.

Reversible secondary pseudohypoaldosteronism due to pyelonephritis

“…The occurrence of secondary PHA has been documented in young infants with urinary tract infection associated with congenital malformations [1,2,3,4,5,6,7,8]. A variety of factors contribute to the pathogenesis of hyponatremia/hyperkalemia in extremely young children, most importantly malformations and renal infection.…”

“…Primary PHA is a hereditary condition that includes at least two clinically and genetically distinct entities and involves either renal or multiple target organ defects. Secondary forms of PHA have been reported rarely, most of which are associated with pyelonephritis secondary to obstructive uropathy or vesicoureteral reflux [1,2,3,4,5,6,7,8]. However, the picture of hyponatremia/hyperkalemia may also be found in infants with acute pyelonephritis, even in the absence of uropathy [9,10,11].…”

Clinical and Molecular Features of Type 1 Pseudohypoaldosteronism

“…PHA3 comprises transient and secondary forms of salt-losing states caused by various pathologies of the kidney, intestinal tract or sweat glands. Nephropathies such as urinary tract infections and obstructive uropathies are the most frequent cause [35,36,37,38]. Contrary to PHA1 and PHA2, the glomerular filtration rate is decreased in these cases.…”

Disturbances of Na/K Balance

“…However, renal causes are encountered more frequently. Nephropathies such as obstructive uropathy (12) or urinary tract infections (13) were reported as causes of transient aldosterone resistance (1). The main characteristic of this type of PHA is a decreased GFR.…”